(IJISMS) Megaureter Management -Five-Year Retrospective Study

International Journal of Innovative Studies in Medical Sciences (IJISMS)

ISSN 2457-063X (Online) www.ijisms.com Volume: 2 Issue: 5 | 2018

Megaureter Management -Five-year Retrospective study

Yasin Idweini MD, PhD. FEBU Senior Consultant and Chairman of Urology, Al Bashir Teaching Hospital- Amman Jordan

peristalsis is seen which transmits abnormal pressures Abstract: up to the kidney, resulting in calyceal dilation. Purpose: Management of patients with megaureter Histologic findings include an excess of circular muscle, (MGU) and differentiating nonobstructive from fibres and collagen in the distal ureter which may obstructive variants, and better defining the indications account for the problem (2). for surgery, remains one of the more challenging dilemmas in Urology. We reviewed our experience in 2. MATERIALS AND METHODS treating megaureter according to the diverse aetiologies Fifteen patients with megaureter were chosen in the of ureteral dilation. period of five years duration. Our cases of mega Materials and methods: 15 patients of megaureter ureters, whether being primary or secondary type, were chosen in the period of five years duration have been separated into three major categories: (Refluxing 7 cases, obstructed 6 cases, and non-refluxing refluxing (7cases), obstructed (6 cases), and non- non-obstructed 2 cases). Male to female ratio was 9:6. refluxing non- obstructed (2 cases). Age (1 month-35 years). Localization was: Lt side 57%, Male to female ratio was 9:6. Age ranged from one Rt. Side 36%, Bilateral 7%. month to 35 years, median 11 years. Localization was: Results: Good results achieved in 87% of patients. Lt Side 57% Rt. Side 36% Bilateral 7%, Patients were investigated during physical examination blood and Conclusions: Precise definition of the cause of the urine analysis, ultrasonography, urography, voiding dilation and proper patient selection may avoid cystogram, Renal Isotopes scan and urodynamic study. complications in the reconstructive surgery. 1) Refluxing megaureter: 7 patients Keywords: megaureter, obstructive, refluxing, A- Primary treatment. -(Primary refluxing megaureter) 2 patients 1. INTRODUCTION B- Secondary The normal ureteral diameter in children is rarely -(Bladder outlet obstruction) 5 patients greater than 5 mm and ureter wider than 7 mm can be -Obstructive Ureterocoele 1 patient considered megaureter (1). The term megaureter could - Urethral stricture 1 patient be applied to any dilated or “big” (mega) ureter. The Paediatric Urology Society in 1976 adopted a standard -post-urethral valves 2 patients nomenclature for categorizing the megalo(wide) ureter -Neuropathic bladder 1 patient that had its basis in the diverse aetiologies of ureteral 2) Obstructed megaureters: 6 patients dilation. The dilated ureter or MGU can be classified A- Primary: into one of four groups based on the cause of the dilation (1) Refluxing (2) Obstructed (3) Both refluxing -Intrinsic obstruction(Ureterocoele) 2 patients and obstructed and (4) Both non-refluxing and non- -Stenosis 1 patient obstructed. Further subdivision into primary or -Adynamic segment 1 patient secondary causes assume additional important for B- Secondary: obvious reasons. A thorough evaluation of the entire urinary tract is required in every case because - Neuropathic bladder 1 patient therapeutic recommendations depend on proper - Retroperitoneal scarring 1 patient categorization. Obstruction at the uretero-vesical 3) Non -refluxing non-obstructed: 2 patients junction is 4 times more common in boys than girls. It A. Primary: is often bilateral but usually asymmetric. The left ureter is slightly more often involved than the right (2). Close -Non-refluxing non-obstructed 1 patient observation either at operation or by fluoroscopy B. Secondary: reveals a failure of the distal ureter to transmit the . Dilatation remaining after relief of distal obstruction. normal peristaltic wave, resulting in a functional obstruction. Moreover, on fluoroscopy, retrograde 1 patient

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3. TREATMENT Regardless of its origin, altered peristalsis prevents the free outflow of urine, and functional obstruction Treatment ranged between remodelling of the ureter results. Secondary obstructive MGU occurs with and reimplantation and was managed expectantly neurogenic and non-neurogenic voiding dysfunction or regarding the aetiology. Ureteral reimplantation intravesical obstruction such as posterior urethral (Politano-Leadbetter technique) with excision of the valves. The dilation that occurs with most of these distal ureter was performed and because of the variants largely resolve once the cause of the elevated excessive dilatation of the ureter, ureteral tapering was intravesical pressures is addressed. In other cases, the necessary. In some cases, nephroureterectomy in ureter remains permanently dilated from what appear dysplastic kidney and non-functioning upper moiety of to be altered compliance or a permanent insult to the the kidney with ureterectomy of refluxing tortuous organ’s peristaltic mechanisms or both. Other severely dilated ureter. Ablation of posterior urethral obstructive causes of ureteral dilation include valves was performed. Medical treatment with ureterocoele, bladder diverticula, neurogenic bladder, anticholinergic drugs was offered to those cases with external compression by retroperitoneal tumour or neuropathic bladder. aberrant vessels. Secondary non-refluxing non- 4. RESULTS obstructed megaureter can result from acute UTI, accompanied by bacterial endotoxins that inhibit Good results were achieved in 87% of patients. peristalsis. Other causes might be lithium toxicity, 5. DISCUSSION diabetes insipidus or mellitus, sickle cell nephropathy and psychogenic polydipsia (11). Where controversy arises is in differentiating nonobstructive variants and better defining the Once reflux, obstruction and secondary causes of indications for surgery. Many urinary tract dilations dilation have been ruled out the designation of primary represent distortions of the collecting system that, non-refluxing nonobstructive MGU is appropriate and although at times quite severe, do not represent an most new-born MGUs, fall in this category (12,13,14). obstructive threat to their associated renal moiety. However, in recent years it has become obvious that at Perinatal ultrasonography has altered the least 50% of cases will have spontaneous resolution. A understanding and management of urologic anomalies period of observation is nearly always appropriate and dilations, with MGUs, being no exception (3). MGU when the diagnosis is made in an asymptomatic comprised 20% of antenatally diagnosed urological patient. Because of the high risk of infection 1-2 years anomalies. a percentage inordinately urinary tract of prophylactic antibiotics are recommended in abnormality, when most were discovered only after neonates (14). A voiding cystourethrogram is an they became symptomatic (e.g. infection calculi) and essential part of the evaluation, not only to rule out surgery were necessary. (4). Today, through prenatal reflux but also to ensure that no abnormality of the detection, most children arrive with abnormalities that lower urinary tract is responsible for the upper urinary are totally asymptomatic. If left undetected many MGUs tract dilation (15). Use of percutaneous renal puncture might never become symptomatic, an observation that is occasionally beneficial in the dilated system, it raises serious questions about treatment. Expectant carries minimal risk, making antegrade urography and treatment and serial ultrasonic follow up studies have pressure-flow studies feasible in selected cases. dramatically redefined the natural history of non- Measurements of the renal pelvis pressure during refluxing MCUs. infusion of saline into the renal pelvis at high It is generally agreed that the cause of primary rates(10ml/min) (the Whitaker test) may help obstructive MGU is an aperistaltic juxta vesical segment differentiate nonobstructive from obstructive dilation 3 to 4 cm long that is unable to propagate urine at (18), unfortunately, there is disagreement; clinical acceptable rates of flow. A variety of histologic and judgment is the final arbiter (17). Our patients were ultrastructural abnormalities that alter function have treated according to the aetiology, we had two patients been described. These include disorientation of muscle with primary refluxing megaureters which were (5,6), muscular hypoplasia, muscular hypertrophy, and treated by ureteral reimplantation with tapering of the mural fibrosis (7). Excess collagen deposition is a dilated ureter. Figure (1-5). common finding (8,9). Ureteric profilometry shows irregular wave patterns within these segments, so called uretero-arrhythmias(10). The distal ureter is usually involved and may be related to arrested development in the musculature of this segment, which is the last portion of the ureter to develop (5).

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treated with ureteropyeloanastomosis to solve the VUR). Unfortunately, the surgeon didn’t identify the presence of obstructive ureterocoele, and he anastomosed incorrectly the refluxing ureter to the obstructive one, which resulted in complicated urolithiasis in the ureterocoele and ipsilateral kidney. Marsupialization of ureterocoele and extraction of big ureteric calculi and reimplantation with tapering of ureter was performed. Lithotripsy was done later for the kidney stone (Figure 6-8) (18).

Fig 1) IVU: Primary Lt. refluxing megaureter Fig2) IVU: Primary refluxing megaureter in infant

Fig 6) KUB: Stone in Rt. Ureterocoele+kidney Fig7) IVU :Rt. Ureterocoele with secondary stone

Fig 3) MCUG: Lt. Refluxing megaureter Fig 4) IVU :(Postoperative) Megaureter corrected

Fig 8) IVU (postoperative) after tapering and reimplantation: Normal urogram The other case of obstructive ureterocoele in duplicated urinary system with non-functioning Fig 5) MCUG (postoperative): Reflux corrected after dysplastic hydronephrotic upper moiety with severely tapering and reimplantation dilated ureter was treated with marsupialization of ureterocoele and heminephroureterectomy (Figure 9- Four other cases with primary obstructed megaureter 13) (19). (adynamic segment, stenosis, ureterocoele) were treated with reimplantation and tapering of ureter. One of the two cases of ureterocoele was a complicated case (18-year old girl was diagnosed 10 years before as a case of VUR in one moiety of duplicated ureter and

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Fig 14) MCUG: Secondary Lt MGU due to posterior urethra valves

Fig 9)IVU Lt obstructive megaureter +Rt. ureterocoele Fig15) MCUG after ablation of valves Fig.10)IVU: Film after 5 hours obstructed MGU The dilation resolved once the cause of the elevated intravesical pressure was addressed. Those cases of bladder outlet obstruction (neuropathic bladder) were treated with anticholinergic drugs. The remaining cases, classified as non-refluxing non-obstructed (Figure16-17) were observed expectantly with serial ultrasonic follow up studies. Good results were achieved in 87% of our cases.

Fig 11)MCUG after marsupialization of ureterocoele:-Rt megaureter+severe hydronephrosis

Fig 16) IVU: Non-refluxing non-obstructed MGU. Fig17) MCUG: No VUR 6. CONCLUSIONS Precise definition of the cause of the dilation and proper patient selection may avoid complications in the reconstructive surgery. Advancement of understanding of the aetiology and pathophysiology of ureteral dilation may play a role in the differentiation of those megaureters that require Fig 12)IVU(postoperative)after marsupilazation+Rt surgery to preserve renal function from those that can heminephroureterectomy: Normal urogram be observed during is transitional period in the organ’s development. Fig 13) MCUG(postoperative) after marsupilazation+Rt heminephroureterectomy: refluxing megaureter When obstruction is suspected, surgery is indicated. corrected Patient selection and surgical technique remain the key Among the remaining cases we had 7 cases of elements to maintain preservation of renal function. secondary megaureters (5 refluxing, 2 obstructive) REFERENCES treated with ablation of posterior urethral valves (Figures 14-15) and optical urethrotomy of stricture [1] Hellstrom M., Hjalmas K., Jacobsson B.et at: urethra. Normalureteral ureteral diameter in infancy and childhood,Acta Radiol 1985; 26: 433.

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